Just returned from the annual CurePSP International Research Symposium, held this year in on the campus of University of California San Francisco on October 27. About 120 researchers attended, many from Europe and Japan. The first keynote speaker was Bruce Miller from UCSF, perhaps the country’s leading behavioral neurologist, who gave an overview of PSP/CBD research with an emphasis on activity of the Tau Consortium, the multi-institutional research group that he directs. The other keynoter was Robert Stern, a neuroscientist at Boston University who directs clinical research at BU’s Chronic Traumatic Encephalopathy Center.
Bruce’s talk touched on many topics — from the nosology and pathology of the various cognitive/behavioral syndromes in the tauopathies to the sleep disturbance in PSP (hyperarousal is common in PSP, while hypoarousal predominates in CBD). Perhaps most interesting was his up-to-the minute summary of the state of tau PET imaging in PSP diagnosis (not nearly ready for prime time, though potential exists).
Bob’s lecture summarized the story of CTE. He emphasized that the most important frequent cause isn’t concussions, but the continual sub-concussive blows to the head such as those suffered by football players during routine blocking and tackling. He was too smart to speculate much about a relationship between the tauopathy of CTE and that of PSP, but I’m not: I’ll say that in both cases, individuals with a genetic predisposition to tau aggregation are exposed to a precipitating factor – repeated brain tissue stretching for one, some sort of toxin for the other. If we can find the genetic background for one, we may find it for the other.
Perhaps the genetic answers will emerge from the whole-exome sequencing project that is complete and in the writing phase or the whole-genome sequencing project that is well under way. But as pointed out in another Symposium talk by Jerry Schellenberg, the U Penn geneticist who heads those efforts for the PSP Genetics Consortium, there’s a lot of “genetic dark matter” in the form of genomic deletions, undetectable by mere sequencing.
Maybe in future posts I’ll get more into the other excellent CurePSP Symposium talks – and the 18 concomitant poster presentations. Or maybe I’ll get distracted by a random shiny object I find somewhere else.
5 thoughts on “PSP by the Bay”
Hi Dr. Golbe – I came across this article on a breakthrough on Huntington’s disease from BBC. I’m curious to know how similar it is to PSP since the article mentions “toxic proteins” in the brain. Could this give new hope to PSP sufferers? http://www.bbc.com/news/health-42308341
Hi, Shaefali — This press release was about an early-phase clinical trial of “silencing RNA” injected into the spinal fluid for Huntington’s disease. It would slow, or (theoretically/hopefully), stop the progression of the damage in the brain. Research in using silencing RNA in PSP has begun, but it has not yet reached human trials. Of course, in PSP, the target would be tau and not huntingtin. So that’s a completely different silencing RNA. But the idea is the same, and I hope it will enter clinical trials in the next couple of years.
I lost my dear Dad to PSP in May of 2017 and am thrilled to learn just now that there are possible treatments in the works because I could find nothing indicative of possible treatments when I researched it for my dad. By the way, we made sure on his death certificate that “cause of death” was PSP because oftentimes, “cause” is listed as one of the symptoms of PSP because it wasn’t correctly diagnosed when the victim was still alive. I urge anyone reading this to make sure psp is listed as the cause, so the statistics are accurate. The more victims, the more likely to gain more research. Lastly, THANK YOU DR GOLBE, for your care and concern for sufferers of this devastating disease! May God be with you.
Sheila, I am sorry to hear that. My dad also has PSP, final stages. I would love to learn more about your experience. Please, let me know if we can email each other. I would love to try and help find a cause/cure for PSP. Such a devastating disease.
Thanks for that comment, Ms. Spalding. Death certificates list the proximate cause and contributory causes. As long as PSP is listed as either of those, it will be accessible to researchers later. If PSP isn’t diagnosed at all during life and an autopsy reveals that diagnosis after the death certificate is completed, an amendment to the certificate can be made. I know that my state, New Jersey, has an easy way for physicians to do this on line and I assume the same is true elsewhere.